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Mitochondrial Inheritance Following Nuclear Transfer: From Cloned Animals to Patients with Mitochondrial Disease

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Somatic Cell Nuclear Transfer Technology

Part of the book series: Methods in Molecular Biology ((MIMB,volume 2647))

Abstract

Mitochondria are indispensable power plants of eukaryotic cells that also act as a major biochemical hub. As such, mitochondrial dysfunction, which can originate from mutations in the mitochondrial genome (mtDNA), may impair organism fitness and lead to severe diseases in humans. MtDNA is a multi-copy, highly polymorphic genome that is uniparentally transmitted through the maternal line. Several mechanisms act in the germline to counteract heteroplasmy (i.e., coexistence of two or more mtDNA variants) and prevent expansion of mtDNA mutations. However, reproductive biotechnologies such as cloning by nuclear transfer can disrupt mtDNA inheritance, resulting in new genetic combinations that may be unstable and have physiological consequences. Here, we review the current understanding of mitochondrial inheritance, with emphasis on its pattern in animals and human embryos generated by nuclear transfer.

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  1. Institute of Animal Breeding and Genetics, University of Veterinary Medicine, Vienna, Austria

    Jörg P. Burgstaller

  2. Departamento de Genética e Evolução, Universidade Federal de São Carlos, São Carlos, Brazil

    Marcos R. Chiaratti

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  1. Chemical Biology Graduate Program, Federal University of São Paulo – UNIFESP, Campus Diadema, Diadema - SP, Brazil

    Marcelo Tigre Moura

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Burgstaller, J.P., Chiaratti, M.R. (2023). Mitochondrial Inheritance Following Nuclear Transfer: From Cloned Animals to Patients with Mitochondrial Disease. In: Moura, M.T. (eds) Somatic Cell Nuclear Transfer Technology . Methods in Molecular Biology, vol 2647. Humana, New York, NY. https://doi.org/10.1007/978-1-0716-3064-8_4

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